ATYPICAL CARCINOID OF THE ESOPHAGUS - A CASE-REPORT AND REVIEW OF THELITERATURE

BACKGROUND. Although carcinoid tumors of the gastrointestinal tract ar e relatively common, their occurrence in the esophagus is exceedingly rare, The authors report a case of an atypical carcinoid presenting in the cervical esophagus of an 82-year-old woman. METHODS. The tumor wa s studied with routine, silver, and immunohistochemical techniques for cytokeratin, chromogranin, and various secretory products. In additio n, the literature was reviewed for carcinoid tumors of the esophagus a nd the findings summarized. RESULTS. The esophageal tumor showed focal necrosis, atypical cytologic features, and increased mitotic activity . It stained diffusely for chromogranin and focally for serotonin; thu s it was considered an atypical carcinoid. The patient was free of dis ease 9 months after excision. On review of the literature, 13 addition al cases of esophageal carcinoid were found. The average age of the pa tients was 60 years with a male predominance of 6:1; the most common p resenting symptoms included dysphagia and weight loss. The majority of tumors occurred in the submucosa of the lower esophagus, and ranged i n size from 1 to 12 cm. All patients except one had surgical treatment , three received adjuvant radiotherapy or chemotherapy. Although follo w-up was limited, survival correlated with stage; seven of ten Stage I or II patients were disease free whereas three of four Stage III or I V patients had died of disease; the fourth patient is alive with brain metastases. CONCLUSIONS. Esophageal carcinoid tumors are exceedingly rare neoplasms. They usually occur in the lower esophagus of males who present with dysphagia. Survival statistics are limited, but appear b est correlated with disease stage. (C) 1997 American Cancer Society.